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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 86 (1993), S. 95-99 
    ISSN: 1432-0533
    Keywords: Polyglucosan body ; Neuropathy ; Demyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In this study we evaluated the relationship between polyglucosan bodies and peripheral nerve lesions. The biopsied sural nerve from a patient with late-onset chronic sensori-motor neuropathy showed many intra-axonal polyglucosan bodies and segmental demyelination/remyelination. The formation of Schwann cell hyperplasia around the demyelinated axons was found at the sites of polyglucosan bodies. These findings suggest that demyelinating neuropathy is a part of the spectrum of the diseases characterized by the accumulation of polyglucosan bodies within cellular compartments.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 86 (1993), S. 547-553 
    ISSN: 1432-0533
    Keywords: Germanium dioxide ; Neurotoxicity ; Demyelinating neuropathy ; Nerve edema
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report and experimental model of germanium dioxide (GeO2)-induced neuropathy in rats. More than 6 months administration of GeO2 to young rats produced neuropathy characterized by segmental demyelination/remyelination and nerve edema. Electron microscopic studies demonstrated that changes in Schwann cells, such as an increased cytoplasmic volume or disintegration of the cytoplasm, were the earliest pathological findings. Schwann cell mitochondria contained high electron-dense materials. Subsequent removal of necrotic Schwann cell debris and myelin by invading macrophages was evident. These findings suggested that the Schwann cells themselves are the primary target of the toxin. The deposition of electron-dense granules in the intra-axonal vesicles, which was suggestive of glycogen granules in mitochondria, was observed in the advanced stage of the neuropathy. The findings of endoneurial edema with splitting of myelin lamellae were noted at the early stage of demyelination. Nerve edema may be the result of GeO2-induced endothelial cell injury.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Key words Immunohistochemistry ; Polymerase chain ; reaction in situ hybridization ; HTLV-I-associated ; myelopathy/tropical spastic paraparesis ; Double ; staining ; Fresh frozen sections
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract HTLV-I-infected cells play an important role in pathogenesis HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP). Our previous studies of quantitative polymerase chain reaction (PCR) and in situ PCR suggested that T cells infiltrating in the spinal cord lesion were infected with HTLV-I. To elucidate the localization of HTLV-I proviral DNA directly, we performed double staining using immunohistochemistry and PCR in situ hybridization (PCR-ISH). Fresh frozen sections of the spinal cord from four HAM patients taken at autopsy were first immunostained with antibodies to pan T cells (UCHL-1), macrophages (KP-1) and helper/inducer T cells (OPD4). Then PCR-ISH was carried out with specific primers and probe for the HTLV-I pX region. UCHL-1-positive cells were noted around perivascular areas and, to some extent, in the parenchyma. Of the UCHL-1-positive cells, 9.4% (case 1), 9.6% (case 2), 1.1% (case 3) and 6.7% (case 4) became positive in HTLV-I PCR-ISH. UCHL-1-negative cells were HTLV-I PCR-ISH negative and almost all KP-1-positive cells were HTLV-I negative. HTLV-I was localized to OPD4-positive cells in examined lesions of cases 2 and 4. These data are a direct demonstration of HTLV-I proviral DNA localizing to infiltrated T cells in HAM/ TSP spinal cord lesions.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0533
    Keywords: Acid maltase deficiency ; Japanese quail ; Early morphological change ; Membrane-bound glycogen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The skeletal muscle of Japanese quails with acid maltase deficiency (AMD) was studied morphologically at various developmental stages, from the 16th embryonal day up to 3 months after hatching. Membrane-bound glycogen particles began to appear in the affected skeletal muscle at the 16th embryonal day. In normal embryonic muscles, a certain amount of free glycogen particles was observed but they were not membrane-bound. Therefore, this is the earliest morphological event in the muscle of Japanese quails with AMD. In muscle at 3 weeks after hatching, the initial focal degeneration of myofibrils was recognizable but it was not associated with autophagic vacuoles. Quails with AMD developed muscle weakness and difficulty in lifting their wings at about 3 months after hatching: then numerous autophagic vacuoles were present. The formation of large autophagic vacuoles followed by fiber loss and fatty replacement seemed ot contribute to the progressive muscle weakness. The study of Japanese quail with AMD will greatly facilitate the elucidation of the pathogenetic mechanism and is also a useful model for therapeutic trials in human AMD.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 82 (1991), S. 55-59 
    ISSN: 1432-0533
    Keywords: Germanium intoxication ; Mitochondrial myopathy ; Ragged-red fiber ; Autophagic vacuole
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The long-term administration of germanium dioxide (GeO2) to rats produced Ge myopathy characterized by the formation of ragged-red fibers. The earliest pathological changes in experimental Ge myopathy were a decrease in cytochrome c oxidase activity and accumulation of high electron-dense materials in mitochondria. These findings suggest that a mitochondrial dysfunction may be most important in the genesis of experimental Ge myopathy, which could be a useful animal model for the investigation of and therapeutic trials for human mitochondrial myopathies.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Adult T cell leukemia ; HTLV-I ; Immunohistochemistry ; In situ polymerase chain reaction ; p53 protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report the pathological changes in skeletal muscle from a patient with acute adult T cell leukemia (ATL). HTLV-I provirus was detected in infiltrating cells using in situ polymerase chain reaction in frozen sections. Furthermore, aberrant expression of the p53 protein was observed in the infiltrating cells. As p53 protein was not observed in mononuclear inflammatory cells in patients with polymyositis, expression of the p53 protein was considered to be one of the characteristic findings in ATL cells. This is the first direct detection of ATL cells in skeletal muscle.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-0533
    Keywords: Key words In situ polymerase chain reaction ; Immunohistochemistry ; Human T cell lymphotropic ; virus type I ; Proviral DNA ; Polymyositis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have analyzed muscle biopsy specimens from polymyositis patients who are also positive for human T cell lymphotropic virus type I (HTLV-I) using both immunohistochemistry for surface antigens of lymphocytes and macrophages and in situ polymerase chain reaction for HTLV-I proviral DNA on the same sections. We found HTLV-I in CD4+ cells but not in macrophages. This finding suggests that most of the HTLV-I-containing CD4+ cells are not macrophages but lymphocytes.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0533
    Keywords: Germanium intoxication ; Vacuolar myopathy ; Mitochondrial abnormality
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Pathological examinations were carried out on the skeletal muscle of a patient with germanium intoxication. The prominent histochemical finding was vacuolar myopathy with lipid excess, increased acid phosphatase activity and decreased cytochrome c oxidase activity. Ultrastructural lesions revealed a mitochondrial abnormality, autophagic vacuoles and accumulation of high electron-dense materials in deformed mitochondria and at the periphery of lipid droplets. Furthermore, the toxic effect of germanium on skeletal muscle was confirmed by the experimentally induced germanium myopathy, which showed autophagic degeneration, decreased cytochrome c oxidase activity and a mitochondrial abnormality with high electron-dense materials.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-0533
    Keywords: Abetalipoproteinemia ; Muscle pathology ; Giant lysosome ; Concentric laminated body
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Pathological examination was carried out of the skeletal muscle of an 8-year-old boy with abetalipoproteinemia. The patient complained of diarrhea, and showed a deficiency of betalipoprotein, decreased fatsoluble vitamins, acanthocytosis and a mild incrase in serum creatine kinase. The prominent histochemical finding was punctate deposits of acid phosphatase activity in most fibers. Ultrastructural lesions revealed a number of giant lysosomes. Although these pathological findings seemed to be related to vitamin E deficiency, other pathological findings such as concentric laminated bodies of filamentous bodies were also observed. The clinical course and the changes in the pathological findings in our patient after long-term vitamin E therapy need to be observed.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-0533
    Keywords: Key words Adult T cell leukemia ; HTLV-I ; Immunohistochemistry ; In situ polymerase chain reaction ; p53 protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report the pathological changes in skeletal muscle from a patient with acute adult T cell leukemia (ATL). HTLV-I provirus was detected in infiltrating cells using in situ polymerase chain reaction in frozen sections. Furthermore, aberrant expression of the p53 protein was observed in the infiltrating cells. As p53 protein was not observed in mononuclear inflammatory cells in patients with polymyositis, expression of the p53 protein was considered to be one of the characteristic findings in ATL cells. This is the first direct detection of ATL cells in skeletal muscle.
    Type of Medium: Electronic Resource
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